Sweet's syndrome belongs to the group of neutrophilic dermatoses. We report the case of a 36-year-old man admitted for stiff neck and fever. He had a history of recurrent oral aphtous ulcers, orchitis, phlebitis, two episodes of febrile acute polyarthritis with interstitial pneumonia. He presented a stiff neck and a temperature of 40 degrees C for two days associated with an erythematonodular eruption of the right periocular region. Laboratory exams showed an inflammatory syndrome with hyperleukocytosis. Skin biopsy showed dermic neutrophilic infiltrates, confirming the diagnosis of Sweet's syndrome. The patient improved dramatically with corticosteroids: the temperature fell and neck stiffness and skin lesions disappeared. In light of this case with a rich cohort of extracutaneous manifestations, we reviewed the literature on the characteristics of Sweet's syndrome. This syndrome is commonly associated with inflammatory and neoplastic diseases.